Management of an acute outbreak of diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in adults from southern Denmark: an observational study

Background Diarrhoea-associated haemolytic uraemic syndrome in adults is a life-threatening, but rare multisystem disorder that is characterised by acute haemolytic anaemia, thrombocytopenia, and renal insufficiency. We aimed to assess the success of management of this disorder with plasma exchange therapy. Methods Patients diagnosed with diarrhoea-associated haemolytic uraemic syndrome in southern Denmark were treated with daily plasma exchange by centrifugation and substitution with fresh frozen plasma. Stool culture and serological testing was done to identify the cause of disease, and the success of management with plasma exchange therapy was assessed from change in platelet count, glomerular filtration rate, and lactate dehydrogenase. Findings During May 25-28, 2011, five patients with a median age of 62 years (range 44-70) presented with diarrhoea-associated haemolytic uraemic syndrome, which was caused by an unusual Shiga-toxin-producing Escherichia coli serotype O104:H4. Strains of E coli showed a high resistance to third-generation cephalosporins because the strains had extended-spectrum beta lactamases. After plasma exchange, median platelet count and glomerular filtration rate increased, median lactate dehydrogenase concentration decreased, and neurological status improved. The time interval from onset of bloody diarrhoea to start of plasma exchange had an inverse correlation with reduction of lactate dehydrogenase concentrations by plasma exchange (p=0.02). All patients were discharged with normal neurological status at 7 days (range 5-8) after starting plasma exchange. Interpretation Early plasma exchange might ameliorate the course of diarrhoea-associated haemolytic uraemic syndrome in adults. However, this finding should be verified in randomised controlled trials